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Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats

8 Nov 2018

Abstract excerpt

Mutations in ubiquilin2 (UBQLN2) have been linked to abnormal protein aggregation in amyotrophic lateral sclerosis (ALS). The mechanisms underlying UBQLN2-related neurodegenerative diseases remain unclear. Using a tetracycline-regulated gene expression system, the ALS-linked UBQLN2 P497H mutant was selectively expressed in either the spinal motor neurons or astrocytes in rats. We found that selectively expressing...

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PMID: 30409191
DOI: 10.1186/s40478-018-0627-9

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